Psoriasis flare-ups in non syndromic autosomal recessive epidermal differentiation disorders

This manuscript reports a cohort of 13 patients with non-syndromic autosomal recessive epidermal differentiation disorders (EDDs) who developed psoriasis flare-ups, either plaque-type or pustular. Eleven of these patients required systemic therapy. Although Th17 pathway activation has previously been shown to be elevated in EDD skin and correlated with erythema and disease severity, therapies targeting the IL-17/IL-23 axis achieved complete control of psoriasis flare-ups in our cohort, while demonstrating minimal efficacy on the underlying EDD. Our findings suggest a shared Th17/23-mediated inflammatory pathway between EDDs and psoriasis and support the relevance of therapeutic strategies targeting this axis in selected patients.